Synonyms
Anterior midline defect; Bladder exstrophy and epispadias complex; Cloacal exstrophy; Congenital malformation
Definition
Bladder exstrophy [BE] is a true congenital malformation characterized by a defect in the closure of the lower abdominal wall and the bladder with consequent eversion of the bladder mucosa through the ventral wall (Siffel et al. 2011).
Clinical Features
Incidence
BE affects about 2.07 per 100,000 births. It may also be associated with cloacal anomalies, other urinary tract and genital defects, particularly epispadias. Some authors consider cloacal exstrophy [CE] as the most severe end of a spectrum of malformations known as bladder exstrophy-epispadias complex (BEEC) or exstrophy-epispadias complex (EEC). CE is likely to result from an earlier development defect than BE.
BE results from a failure of mesenchymal migration between the ectodermal and endodermal layers of the cloacal membrane, normally occurring between the 6th and 7th week of development and...
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References and Further Reading
Corica, F. A., Husmann, D. A., Churchill, B. M., et al. (1997). Intestinal metaplasia is not a strong risk factor for bladder cancer: Study of 53 cases with long-term follow-up. Urology, 50, 427–431.
Husmann, D. A., & Rathbun, S. R. (2008). Long-term follow up of enteric bladder augmentations: The risk for malignancy. Journal of Pediatric Urology, 4, 381–385.
Junior, R. E., Leaf, E. M., Zhang, D., et al. (2010). Fibroblast growth factor-10 signals development of von Brunn’s nests in the exstrophic bladder. American Journal of Physiology. Renal Physiology, 299, 1094–1110.
Kathopoulis, N., Thomakos, N., Mole, I., Papaspirou, I., Ntai, S., & Rodolakis, A. (2016). Anterior pelvic exenteration for exstrophic bladder adenocarcinoma: Case report and review. International Journal of Surgery Case Reports, 25, 13–15.
Rubenwolf, P. C., Eder, F., Ebert, A. K., Hofstaedter, F., Woodhouse, C. R. J., & Roesch, W. H. (2013). Persistent histological changes in the exstrophic bladder after primary closure – A cause for concern? The Journal of Urology, 189, 671–677.
Siffel, C., et al. (2011). Bladder exstrophy: An epidemiologic study from the international clearinghouse for birth defects surveillance and research, and an overview of the literature. American Journal of Medical Genetics. Part C, Seminars in Medical Genetics, 157, 321–332.
Smeulders, N., & Woodhouse, C. (2001). Neoplasia in adult exstrophy patients. British Journal of Urology, 87, 623–628.
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Henriques, V., Raspollini, M.R., Lopez-Beltran, A. (2020). Bladder Cloacal Extrophy. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4786
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4786
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